Five‐year survival without major disability of extremely preterm infants born at 22–27 weeks’ gestation admitted to a NICU

Aim: To compare the 5‐year survival without major disability in infants born at the threshold of viability at 22–25 weeks who were actively treated in the delivery room and admitted to a NICU to that of those born at 26–27 weeks of gestation. Methods: All infants between 22⁺⁰ and 27⁺⁶ weeks of gestation admitted to a regional intensive care unit during 1999–2003 were enroled prospectively. The survival and major disability at 5 years of age were analysed by gestational age. Results: Of 242 treated infants, 202 survived (83.5%). Although the overall survival rate was significantly higher in the 25–27 weeks’ gestation infants than the 22–24 weeks’ gestation infants (p < 0.001), the survival rate among infants 22–24 weeks (63.6%, 63.6%, and 70%) did not significantly differ, likewise infants 25–27 weeks (88.7%, 90.6%, and 92%) had similar results. Overall, 28 children (14.4% of assessed) had major disability. Both survival and survival without major disability were positively influenced by increasing gestational age, increasing birth weight, being born at 25–27 weeks and being female child. Conclusion: With an active approach in treatment, the outcome of infants born at 25 weeks is comparable to those born at 26–27 weeks. Thus, the ‘grey zone’ in which the risk of adverse outcome is high narrows to 22–24 weeks.     

An Inhalation Device for Exposing Eats to Cigarette Smoke

With an inhalation device for exposing small animals to fresh cigarette smoke, more accurate duplication of the physical and chemical characteristics of human cigarette smoking is achieved than is possible with the devices or chambers described in the literature. The reproducibility of the device was tested by measuring the polonium 210 deposition on filters and in the lungs of rats when labeled cigarettes were smoked by the device. With minor modifieations, the device would be usable for a variety of multistress inhalation exposures and for any of several species of small animals.     

Kabuki syndrome: description of dental findings in 8 patients

The cardinal features of Kabuki (Niikawa-Kuroki) syndrome (KS) include characteristic facial dysmorphic features, mild to moderate mental deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and postnatal growth retardation. We identified 8 patients with KS in a genetics clinic over the past 5 years. All were Caucasians, except for 2 who were of mixed Aboriginal and Caucasian descent. All had the facial gestalt, the dermatoglyphic abnormalities characteristic of the syndrome, and developmental delay. Dental abnormalities of permanent teeth were seen in all 8 cases; 6 had missing lower incisors. Five patients had uniquely abnormal upper incisor teeth shape; the upper incisors had a 'flat head' screwdriver-shaped appearance. Other dental abnormalities included missing lower lateral incisors, missing second premolars, and ectopic upper 6-year molars. We believe the presence of the unique dental findings will prove useful in the diagnostic assessment of individuals with KS.     

Quantity not composition of dietary fats represents the dominant contributor to experimental obesity: Relevance to human pathophysiology

Plant fats are low in saturated fats but high in unsaturated fats compared to animal fats, and are supposedly less obesogenic. This study compared the obesogenic effects of plant and animal derived fatty diets in Wistar rats. Rats of each gender were divided into three dietary (standard chow (SC), high fat diet rich in animal fat (HFDaf) and a high fat diet rich in plant fat (HFDpf)) groups of ten each and fed for 17 weeks. Anthropometric, Adiposity and nutritive variables were assessed using standard methods. Comparing HFDpf to HFDaf: Abdominal circumference (AC),initial feed intaken (IFI), final feed intake(FFI), final body weight (FBW), white adipose tissue (WAT) were increased but brown adipose tissue (BAT) decreased in male rats fed with HFDpf; also, there were increased body length, IFI, FFI but decreased AC, FBW, BAT in female rats fed with HFDpf. Comparing male to female rats: Thoracic circumference, IFI, FFI, energy intake were increased while Adiposity index decreased across diet groups in male rats; the AC, FBW increased while WAT, BAT decreased in HFDpf fed group, also, BAT was increased but AC, FBW decreased in HFDaf fed group in male rats. Palatability and high feed efficiency of consumed diets were more associated with obesogenic risk than just the level of saturation. Therefore, Obesogenic effects of fatty diets in both genders is more dependent on the quantity (amount) of fatty diet consumed than the dietary fat composition alone.     

A Community Breast Center Report Card Determined by Participation in the National Quality Measures for Breast Centers Program

Abstract: Measurement of quality indicators and peer comparison has been demonstrated to improve quality of care. The goal of this study was to determine whether a community breast center, in collaboration with the National Consortium of Breast Centers (NCBC), could voluntarily audit the quality of breast cancer care, confidentially transmit quality information to the NCBC, and receive peer performance comparisons. Quality indicator metrics from consecutive breast cancer patients undergoing care at a community interdisciplinary breast center were entered into a prospective database of quality measures that were defined by the NCBC. Retrospective review of patients from 2004 to 2006 was performed and subsequent quality indicator data was submitted electronically to the NCBC National Quality Measures for Breast Centers (NQMBC^TM) program. The percentage of new cancer diagnoses made by needle biopsy techniques was 94%, 95% and 96% from 2004 to 2006. Sentinel lymph node utilization in eligible patients was 93%, 96% and 91% from 2004 to 2006 and the immediate intraoperative pathologic frozen section false negative rate of the sentinel lymph node was 6.5%, 4.7% and 4%. Chart documentation of “patient participation in shared decision making for breast conserving therapy versus mastectomy” improved from 74% to 99% (p < 0.05) from 2004 to 2006. Adjuvant systemic treatment for stage 2 breast cancer occurred in 76%, 89% and 77% of patients from 2004 to 2006. Neutropenia requiring hospital admission occurred in no patients in 2004 but in 4.8% and 2.9% in 2005 and 2006. The re‐excision lumpectomy rates for stage 0, 1, 2, and 3 breast cancer patients from 2004 to 2006 was 14.2%, 22% and 24.8%. Quality indicator data was submitted to the NQMBC^TM with successful confidential receipt of peer performance comparisons. Voluntary interdisciplinary institutional audits of breast cancer quality can be successfully submitted to the NQMBC^TM with confidential peer performance comparison.     

Temporomandibular Pain Dysfunction: Can Electrotherapy Help?

Temporomandibular pain dysfunction syndrome (TMPDS) comprises a variety of signs and symptoms which may be present in any combination, dependent upon the progress and stage of the disorder. The diagnostic criteria are pain on palpation of the temporomandibular joint (TMJ), muscle tenderness on examination, joint sounds, and limitation or deviation of jaw movement on opening. Headache may also be a symptom. Physiotherapy is commonly used to treat this disorder but there is little published material in the field of clinical trials comparing different methods of physiotherapy. Most reports are anecdotal. This paper reports four different physiotherapy modalities in the management of TMPDS when compared with a placebo group. Short-wave diathermy, megapulse, ultrasound and soft laser were tested. There was no statistically significant different in success rate between any of the four methods but each was significantly better than placebo treatment.     

Expression of the Tn antigen in myelodysplasia, lymphoma, and leukemia

Expression of the normally cryptic blood group antigen Tn has occasionally been reported in hematologic disease, but the true frequency of this change is not known. A mouse monoclonal antibody (FBT3) and immunohistochemistry were used to examine expression of the Tn antigen. Expression was not detected in 35 normal bone marrow aspirates examined, but it was detected in 5 of 725 abnormal bone marrow aspirates, including 2 (3.6%) of 55 cases of de novo acute nonlymphocytic leukemia and 2 cases that terminated in acute nonlymphocytic leukemia. In two patients, one with acute myeloblastic leukemia and the other in blast transformation of chronic myeloid leukemia, the Tn antigen was expressed on 2 percent of blast cells. In one case of non-Hodgkin's lymphoma, 4 percent of normal myeloid cells expressed the antigen. In the other two cases, one of acute myelomonocytic leukemia and the other of myelodysplasia, only 2 to 8 percent of myeloid and erythroid cells initially were Tn positive. Subsequent serial immunohistochemical studies of bone marrow aspirates and peripheral blood in these two cases showed increasing numbers of Tn-positive erythroid and myeloid cells 8 to 12 months before polyagglutination was detected serologically. Tn-positive cells increased to > 90 percent in the terminal phase in both cases of both diseases. The results suggest that Tn expression in these two patients may have conferred a growth advantage to the cells and could be related to disease progression.     

Genetically defined autoinflammatory diseases

Autoinflammatory diseases are hyperinflammatory, immune dysregulatory conditions that typically present in early childhood with fever and rashes and disease‐specific patterns of organ inflammation. This review provides a historic background of autoinflammatory disease research, an overview of the currently genetically defined autoinflammatory diseases, and insights into treatment strategies derived from understanding of the disease pathogenesis. The integrative assessment of autoinflammatory conditions led to the identification of innate pro‐inflammatory cytokine ‘amplification loops’ as the cause of the systemic and organ‐specific disease manifestations, which initially centered around increased IL‐1 production and signaling. More recently, additional innate pro‐inflammatory cytokine amplification loops resulting in increased Type I IFN, IL‐17, IL‐18, or IL‐36 signaling or production have led to the successful use of targeted therapies in some of these conditions. Clinical findings such as fever patterns, type of skin lesions, genetic mutation testing, and the prevalent cytokine abnormalities can be used to group autoinflammatory diseases.